ACR Meeting Abstracts

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  • Abstract Number: 1101 • 2018 ACR/ARHP Annual Meeting

    Extracellular Vesicle-Mediated Delivery of EBV SMALL RNA (EBER1) Activates LUPUS Nephritis Related Antiviral Immunity in Tubular Epithelial CELLS VIA TLR3

    R Baglio1, M Tsang-A-Sjoe2, M Eijndhoven1, K Jordanova3, N Groenewegen1, J van Weering4, S Verkuijlen1, Irene E.M. Bultink5, J Middeldorp6, J Roelofs7, Alexandre Voskuyl8 and M Pegtel1, 1pathology, VU University medical center, Amsterdam, Netherlands, 2Rheumatology, Amsterdam Rheumatology and immunology Center | VU University Medical Center, Amsterdam, Netherlands, 3gynaecologic oncology, VU University medical center, Amsterdam, Netherlands, 4functional genomics, VU University medical center, Amsterdam, Netherlands, 5Rheumatology, Amsterdam Rheumatology and immunology Center | VU University Medical Center, Amsterdam, Netherlands, Amsterdam, Netherlands, 6pathology, VU Uninersity medical center, Amsterdam, Netherlands, 7pathology, Amsterdam Medical Center, Amsterdam, Netherlands, 8Amsterdam Rheumatology and immunology Center, Location VU University Medical Center, Amsterdam, Netherlands

    Background/Purpose: In lupus nephritis (LN), genetic and environmental factors drive the chronic activation of antiviral defenses leading to immune complex-mediated glomerular and tubular damage. Increasing…
  • Abstract Number: 1102 • 2018 ACR/ARHP Annual Meeting

    Multi-Organ RNA-Sequencing of Patients with Systemic Sclerosis (SSc) Finds That Intrinsic Subsets Are Conserved across Organ Systems

    Bhaven K. Mehta1, Jennifer Franks2, Yue Wang1, Guoshuai Cai2, Diana M. Toledo3, Tammara A. Wood2, Kimberly A. Archambault1, Noelle Kosarek1, Kathleen D. Kolstad4, Marianna Stark5, Antonia Valenzuela6, David Fiorentino7, Nielsen Fernandez-Becker8, Laren Becker8, Linda Nguyen9, John Clarke10, Francesco Boin11, Paul Wolters12, Lorinda Chung13 and Michael L. Whitfield14, 1Molecular and Systems Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, 2Department of Molecular and Systems Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, 3Department of Molecular & Systems Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, 4Rheumatology, Stanford University Medical Center, Stanford, CA, 5Stanford University, Stanford, CA, 6Immunology and Rheumatology, Stanford University, Palo Alto, CA, 7Dermatology, Stanford University School of Medicine, Stanford, CA, 8Gastroenterology, Stanford University School of Medicine, Palo Alto, CA, 9Gastroenterology & Hepatology, Stanford University School of Medicine, Palo Alto, CA, 10Gastroenterology, Stanford University School of Medicine, Stanford, CA, 11Rheumatology, University California, San Francisco, San Francisco, CA, 12Pulmonary Division, Department of Medicine, University of California, San Francisco, San Francisco, CA, 13Immunology and Rheumatology, Stanford University School of Medicine, Palo Alto, CA, 14Biomedical Data Science, Geisel School of Medicine at Dartmouth, Hanover, NH

    Background/Purpose: Internal organ involvement is the primary cause of morbidity and mortality in systemic sclerosis (SSc).  Here we tested the hypothesis generated from a meta-analysis…
  • Abstract Number: 1103 • 2018 ACR/ARHP Annual Meeting

    Systemic Sclerosis Has a Distinct Serum Protein Profile That Correlates with Its Clinical Manifestations

    Chiara Bellocchi1,2, Jun Ying3, Ellen Goldmuntz4, Lynette Keyes-Elstein5, John Varga6, Monique Hinchcliff6, Peter McSweeney7, Daniel E. Furst8, Richard Nash7, Leslie Crofford9, Beverly Welch10, Ashley Pinckney5, Maureen D. Mayes11,12, Keith Sullivan13 and Shervin Assassi12, 1University of Texas Health Science Center at Houston, Houston, TX, 2Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy, 3Department of Internal Medicine - Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, 4NIAID, National Institutes of Health, Bethesda, MD, 5Rho Federal Systems, Inc., Chapel Hill, NC, 6Northwestern University, Chicago, IL, 7Colorado Blood Cancer Institute, Denver, CO, 8University of California Los Angeles, Los Angeles, CA, 9Division of Rheumatology and Immunology, Vanderbilt University Medical Center, Nashville, TN, 10National Institutes of Health, Bethesda, MD, 11Internal Medicine/Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, 12Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, 13Duke University Medical Center, Durham, NC

    Background/Purpose: Proteomic studies with an extensive panel of measured proteins are still scarce in systemic sclerosis (SSc). The Scleroderma: Cyclophosphamide or Transplantation (SCOT) trial showed…
  • Abstract Number: 1104 • 2018 ACR/ARHP Annual Meeting

    Mass Cytometry Analysis Detects Dysregulated T Cell Complement Responses in Diffuse Cutaneous Systemic Sclerosis

    Giuseppina Arbore1, Shahram Kordasti2, Claudia Kemper3,4,5, Dennis Hourcade6, Benedetta Costantini2, Leo Placais3, Lynne Mitchell6, Richard Ellis4, Christopher P. Denton7, David Abraham8 and Voon H. Ong8, 1Division of Immunology, Transplantation and Infectious Diseases, San Raffaele Scientific Institute, Milano, Italy, 2Systems Cancer Immunology Lab, King's College London, London, United Kingdom, 3Laboratory of Molecular Immunology and the Immunology Center, National Institutes of Health, Washington, WA, 4School of Immunology and Microbial Sciences, King's College London, London, United Kingdom, 5Institute for Systemic Inflammation Research, University of Lübeck, Lübeck, Germany, 6Division of Rheumatology, Washington University School of Medicine, St Louis, WA, 7UCL Division of Medicine, Royal Free Campus, London, United Kingdom, 8Division of Medicine, University College London, London, United Kingdom

    Background/Purpose: Aberrant CD4+ T cell activation is implicated in disease progression in systemic sclerosis (SSc). Activated T cells release various cytokines that may drive inflammation,…
  • Abstract Number: 1105 • 2018 ACR/ARHP Annual Meeting

    Abatacept Is Effective in Experimental Digestive and Lung Tissue Fibrosis

    Gonçalo Boleto1, Christophe Guignabert2, Sonia Pezet3, Anne Cauvet3, Jérémy Sadoine4, Ly Tu2, Carole Nicco3, Camille Gobeaux5, Frederic Batteux3, Yannick Allanore3 and Jérôme Avouac1, 1Université Paris Descartes, Sorbonne Paris Cité, Service de Rhumatologie A, Hôpital Cochin, Paris, France, Paris, France, 2INSERM UMR_S 999, Le Plessis-Robinson, France, Le Plessis-Robinson, France, 3Université Paris Descartes, Sorbonne Paris Cité, INSERM U1016, Institut Cochin, CNRS UMR8104, Paris, France, Paris, France, 4EA 2496 Pathologie, Imagerie et Biothérapies Orofaciales, UFR Odontologie, Université Paris Descartes and PIDV, PRES Sorbonne Paris Cité, Montrouge, France, Montrouge, France, 5Clinical Chemistry Laboratory, Cochin and Hôtel-Dieu Hospitals, Paris, France, Paris, France

    Background/Purpose: A previous report showed that abatacept (IgG-CTLA-4) prevented and induced regression of inflammation-driven dermal fibrosis in two different mouse models of systemic sclerosis (SSc)…
  • Abstract Number: 1106 • 2018 ACR/ARHP Annual Meeting

    Signal Transducer and Activator of Transcription 3 (STAT3) Activation in Peripheral Blood Mononuclear Cells of Systemic Sclerosis Patients: Correlation with Disease Specific Manifestations

    Fabio Cacciapaglia1, Simone Perniola2,3, Livio Urso1, Emanuela Praino4, Rita Bizzoca2, Dorotea Natuzzi2, Nunzia Lacarpia2 and Florenzo Iannone5, 1Department of Emergency and Organs Transplantation (DETO); University of Bari (Italy), Rheumatology Unit - D.E.T.O. - University of Bari (ITALY), Bari, Italy, 2Rheumatology Unit - D.E.T.O. - University of Bari (ITALY), Bari, Italy, 3Department of Verona - University of Verona (ITALY), Verona, Italy, 4DIM, Rheumatology Unit - D.E.T.O. - University of Bari (ITALY), Bari, Italy, 5D.E.T.O., Rheumatology Unit - D.E.T.O. - University of Bari (ITALY), Bari, Italy

    Background/Purpose: Signal transducer and activator of transcription 3 (STAT3) is a transcription factor regulated by phosphorylation of key tyrosine residues that regulates pivotal cell processes…
  • Abstract Number: 1107 • 2018 ACR/ARHP Annual Meeting

    Expression Quantitative Trait Loci -eQTL- Analysis in Systemic Sclerosis

    Martin Kerick1, David Gonzalez Serna1, Maria Teruel2, Sepideh Babaei3, Marialbert Acosta-Herrera1, Elena Carnero-Montoro2, Zuzanna Makowska3, Anne Buttgereit4, Guillermo Barturen2, Sikander Hayat4, Jorge Kageyama4, Manuel Martínez-Bueno2, PRECISESADS Clinical Consortium5, Ralf Lesche3, Marta Alarcón-Riquelme2 and Javier Martin1, 1Institute of Parasitology and Biomedicine López-Neyra, IPBLN-CSIC, Granada, Spain, 2Medical Genomics, Center for Genomics and Oncological Research (GENYO), Granada, Spain, 3Pharmaceuticals Division, Bayer Pharma Aktiengesellschaft, Berlin, Germany, 4Bayer Pharma Aktiengesellschaft, Berlin, Germany, 5Spain

    Background/Purpose: Systemic Sclerosis (SSc) is a complex systemic autoimmune disorder characterized by fibrosis of the skin and internal organs. Genetics and environmental factors contribute both…
  • Abstract Number: 1108 • 2018 ACR/ARHP Annual Meeting

    Antibody Repertoire Dynamics in Systemic Sclerosis after Myeloablative Autologous Hematopoietic Stem-Cell Transplantation or Cyclophosphamide Treatment

    Julia Z. Adamska1,2, Leslie Crofford3, Daniel E. Furst4,5, Ellen Goldmuntz6, Lynette Keyes-Elstein7, Maureen D. Mayes8, Peter McSweeney9, Richard Nash9, Ashley Pinckney10, Beverly Welch11, Keith Sullivan12 and William H. Robinson1,2, 1Stanford University, Stanford, CA, 2VA Palo Alto Health Care System, Palo Alto, CA, 3Division of Rheumatology and Immunology, Vanderbilt University Medical Center, Nashville, TN, 4University of California Los Angeles, Los Angeles, CA, 5University of Washington, Seattle, WA, 6NIAID, NIH, Bethesda, MD, 7Clinical Statistics, Rho Federal Systems, Inc., Chapel Hill, NC, 8Rheumatology, University of Texas McGovern Medical School, Houston, TX, 9Colorado Blood Cancer Institute, Denver, CO, 10Rho Federal Systems, Inc., Chapel Hill, NC, 11National Institutes of Health, Bethesda, MD, 12Duke University Medical Center, Durham, NC

    Background/Purpose: Myeloablative autologous hematopoietic stem-cell transplantation (HSCT) was recently demonstrated to provide benefit over monthly cyclophosphamide (CYC) in the treatment of diffuse cutaneous systemic sclerosis…
  • Abstract Number: 1109 • 2018 ACR/ARHP Annual Meeting

    Gene Expressions of TMEM176A and TMEM176B Were Prominent at the Stage of Subclinical Pulmonary Vascular Disease in Systemic Sclerosis

    Yoshinobu Koyama1, Soichiro Fuke2, Takashi Ohno3, Yoshiharu Sato4 and Toshie Higuchi1, 1Center for Autoimmune Diseases, Division of Rheumatology, Japan Red Cross Okayama Hospital, Okayama, Japan, 2Department of Cardiology, Japan Red Cross Okayama Hospital, Okayama, Japan, 3Management of medical safety, Okayama University Hospital, Okayama, Japan, 4DNA Chip Research Inc, Tokyo, Japan

    Background/Purpose: Pulmonary arterial hypertension (PAH) is prominent as a vascular involvement of systemic sclerosis (SSc), which remains a leading cause of death in spite of…
  • Abstract Number: 1110 • 2018 ACR/ARHP Annual Meeting

    Characterization of CD20+ T Cells in Patients with Systemic Sclerosis

    Monica D'Orazio1, Elisabeth Fliesser2, Florentine Moazedi-Fürst3, Martin Stradner4, Sonja Kielhauser3, Johannes Fessler5 and Hans-Peter Brezinschek1, 1Internal Medicine/Division of Rheumatoloy and Immunology, Rheumatology and Immunology, Medical University Graz, Austria, Graz, Austria, 2Div. of Rheumatology and Immunology, Medical University of Graz, Graz, Austria, 3Rheumatology and Immunology, Medical University Graz, Austria, Graz, Austria, 4Department of Rheumatology and Immunology, Medical University of Graz, Graz, Austria, 5Department of Neurology, Harvard Medical School, Boston, MA

    Background/Purpose: Recently, it has been demonstrated that a subset of T cells expresses the B-cell marker CD20. It has been postulated that in rheumatoid arthritis…
  • Abstract Number: 1111 • 2018 ACR/ARHP Annual Meeting

    CBS004, a Novel Monoclonal Antibody Against Bdca-2 Inhibits TLR-Induced Activation of Human pDC in Vitro and In Vivo. a Novel Therapeutic Target for Systemic Sclerosis

    Clarissa Corinaldesi1, Yasser M El-Sherbiny2, Gemma Migneco3, Rebecca Ross1, Steve Holmes4, Clive McKimmie5 and Francesco Del Galdo1, 1Leeds Biomedical Research Centre and Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom, 2NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals, NHS Trust, Leeds, United Kingdom, 3Institute of Cancer and Pathology, University of Leeds, Leeds, United Kingdom, 4Capella Biosciences, LTD, London, United Kingdom, 5Virus Host Interactions Team, Section of Infection and Immunity, University of Leeds, Leeds, United Kingdom

    Background/Purpose: Human plasmacytoid dendritic cells (pDCs) have been implicated in the pathogenesis of Systemic Sclerosis (SSc) through their ability to infiltrate the skin and secrete…
  • Abstract Number: 1112 • 2018 ACR/ARHP Annual Meeting

    Downregulated Expression of Interferon Regulatory Factor 8 in Circulating Monocytes Exhibits Pro-Fibrotic Phenotype in Patients with Systemic Sclerosis

    Yasushi Ototake1, Yukie Yamaguchi1, Miho Asami1, Noriko Ikeda1, Daisuke Kurotaki2, Tomohiko Tamura2 and Michiko Aihara1, 1Department of Environmental Immuno-Dermatology, Yokohama City University Graduate School of Medicine, Yokohama, Japan, 2Department of Immunology, Yokohama City University Graduate School of Medicine, Yokohama, Japan

    Background/Purpose: Systemic sclerosis (SSc) is a multisystem connective tissue disease characterized by autoimmunity, vasculopathy, and excessive organ fibrosis. Although the etiology of the disease is…
  • Abstract Number: 1113 • 2018 ACR/ARHP Annual Meeting

    Distinct Pathways in Anti-RNP-Associated Pulmonary Hypertension and Anti-RNP-Associated Raynaud’s Phenomenon

    Lisa Harlow1, Ying Wang2, YunJuan Zang2, Vineet Gupta3, Karen Young4, Jian Huang4, Bethly Aubourg2 and Eric Greidinger5, 1Rheumatology and Clinical Immunology, University of Miami Miller School of Medicine, Miami, FL, 2Rheumatology, University of Miami, Miami, FL, 3Rush University, Chicago, IL, 4Pediatrics, University of Miami, Miami, FL, 5Miami VA Medical Center, Miami, FL

    Background/Purpose: Anti-RNP Autoimmunity is associated with multiple manifestations of vasculopathy, including Raynaud’s Phenomenon (RP) and Pulmonary Hypertension (PH). We have previously linked anti-K10 antibodies to…
  • Abstract Number: 1114 • 2018 ACR/ARHP Annual Meeting

    Proteomic Aptamer Analysis Reveals a Distinct Profile of Very Early Systemic Sclerosis (SSc) Patients at Risk for Progression Toward Definite SSc

    Chiara Bellocchi1, Shervin Assassi2, Jun Ying3, Chandra Mohan4, Alessandro Santaniello1 and Lorenzo Beretta1, 1Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy, 2University of Texas McGovern Medical School, Houston, TX, 3Department of Internal Medicine - Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, 4Department of Biomedical Engineering, University of Houston, Houston, Texas, USA, Houston, TX

    Background/Purpose: To determine via SOMAscan aptamer proteomic analysis (>1200 proteins analyzed) the factors associated with disease transition from very early systemic sclerosis (EaSSc) to definite…
  • Abstract Number: 1115 • 2018 ACR/ARHP Annual Meeting

    In Vivo Assessment of Prevention of Lung Fibrosis Using the Pan-PPAR Agonist Lanifibranor in the Tβriiδk-Fib Mouse Model of Systemic Sclerosis

    Emma C. Derrett-Smith1,2, Shiwen Xu1, David Abraham3, Olivier Lacombe4, Pierre Broqua4, Jean-Louis Junien4, Irena Konstantinova5 and Christopher P. Denton6, 1Division of Medicine, University College London, London, United Kingdom, 2Rheumatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom, 3Division of Medicine, Centre for Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom, 4Inventiva, DAIX, France, 5Inventiva, Daix, France, 6University College London, London, United Kingdom

    Background/Purpose: The TbRIIDk-fib transgenic (TG) mouse model of scleroderma carries a fibroblast-specific transforming growth factor β (TGFβ) receptor II mutation resulting in balanced up-regulation of…
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